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Recently, it was found that polyQ aggregates accumulate as a result of vaccinia\u2010related kinase 2 (<jats:styled-content style=\"fixed-case\">VRK<\/jats:styled-content>2)\u2010mediated degradation of <jats:styled-content style=\"fixed-case\">TCP<\/jats:styled-content>\u20101 ring complex (<jats:styled-content style=\"fixed-case\">TR<\/jats:styled-content>iC)\/chaperonin\u2010containing <jats:styled-content style=\"fixed-case\">TCP<\/jats:styled-content>\u20101 (<jats:styled-content style=\"fixed-case\">CCT<\/jats:styled-content>), which has an essential role in the prevention of polyQ protein aggregation and cytotoxicity. The levels of <jats:styled-content style=\"fixed-case\">VRK<\/jats:styled-content>2 are known to be much higher in actively proliferating cells but are maintained at a low level in the brain via an unknown mechanism. Here, we found that basal levels of neuronal cell\u2010specific <jats:italic><jats:styled-content style=\"fixed-case\">VRK<\/jats:styled-content>2 <\/jats:italic><jats:styled-content style=\"fixed-case\">mRNA<\/jats:styled-content> are maintained by post\u2010transcriptional, rather than transcriptional, regulation. Moreover, heterogeneous nuclear ribonucleoprotein Q (<jats:styled-content style=\"fixed-case\">HNRNP<\/jats:styled-content> Q) specifically binds to the 3\u02b9untranslated region of <jats:italic><jats:styled-content style=\"fixed-case\">VRK<\/jats:styled-content>2 <\/jats:italic><jats:styled-content style=\"fixed-case\">mRNA<\/jats:styled-content> in neuronal cells to reduce the <jats:styled-content style=\"fixed-case\">mRNA<\/jats:styled-content> stability. As a result, we found a dramatic decrease in <jats:styled-content style=\"fixed-case\">CCT<\/jats:styled-content>4 protein levels in response to a reduction in <jats:styled-content style=\"fixed-case\">HNRNP<\/jats:styled-content> Q levels, which was followed by an increase in polyQ aggregation in human neuroblastoma cells and mouse cortical neurons. Taken together, these results provide new insights into how neuronal <jats:styled-content style=\"fixed-case\">HNRNP<\/jats:styled-content> Q decreases <jats:italic><jats:styled-content style=\"fixed-case\">VRK<\/jats:styled-content>2 <\/jats:italic><jats:styled-content style=\"fixed-case\">mRNA<\/jats:styled-content> stability and contributes to the prevention of Huntington's disease, while also identifying new prognostic markers of HD.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p>\n<jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc14638-fig-0005-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text>\n<\/jats:p><\/jats:sec>","DOI":"10.1111\/jnc.14638","type":"journal-article","created":{"date-parts":[[2018,11,29]],"date-time":"2018-11-29T09:18:28Z","timestamp":1543483108000},"page":"413-426","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["HNRNP Q suppresses polyglutamine huntingtin aggregation by post\u2010transcriptional regulation of vaccinia\u2010related kinase 2"],"prefix":"10.1111","volume":"149","author":[{"given":"Hye Guk","family":"Ryu","sequence":"first","affiliation":[{"name":"Department of Life Sciences Pohang University of Science and Technology (POSTECH) Pohang Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sangjune","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Life Sciences Pohang University of Science and Technology (POSTECH) Pohang Korea"},{"name":"Neuroregeneration and Stem Cell Programs Institute for Cell Engineering Baltimore Maryland USA"},{"name":"Department of Neurology The Johns Hopkins University School of Medicine Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saebom","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Life Sciences Pohang University of Science and Technology (POSTECH) Pohang Korea"},{"name":"Neuroregeneration and Stem Cell Programs Institute for Cell Engineering Baltimore Maryland USA"},{"name":"Department of Neurology The Johns Hopkins University School of Medicine Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eunju","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Integrative Biosciences and Biotechnology Pohang University of Science and Technology (POSTECH) Pohang Korea"},{"name":"Advanced Bio Convergence Center Pohang Technopark Pohang Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyo\u2010Jin","family":"Kim","sequence":"additional","affiliation":[{"name":"Division of Integrative Biosciences and Biotechnology Pohang University of Science and Technology (POSTECH) Pohang Korea"},{"name":"SL BIGEN Seongnam Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Do\u2010Yeon","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Life Sciences Pohang University of Science and Technology (POSTECH) Pohang Korea"},{"name":"Department of Pharmacology School of Dentistry Kyungpook National University Daegu Korea"},{"name":"Brain Science &amp; 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